What changed: Adult T-cell leukaemia/lymphoma

When this page itself was last checked or edited.

HTLV-1 serology belongs in the work-up of any T-cell lymphoma or leukaemia in a person who comes from, or whose parents come from, south-western Japan, the Caribbean, west or central Africa, parts of South America, Iran or Romania. Without the test the disease is reported as peripheral T-cell lymphoma not otherwise specified and treated on a pathway that does not fit it. A positive test is followed by confirmation that the virus is clonally integrated in the tumour cells, because asymptomatic infection is common in those populations and does not by itself mean lymphoma.

Three things that belong to this disease and not to the rest of the family. Calcium, which can be very high because the tumour makes parathyroid hormone-related protein, and which may present as confusion, thirst or kidney failure before the lymphoma is recognised. Strongyloides stercoralis, because the immune suppression caused by the virus allows hyperinfection, which is fatal and is prevented by screening and treating before immunosuppressive therapy. And the central nervous system, which is commonly involved in the aggressive types and is examined by lumbar puncture.

The revised report added the classification of cutaneous disease, disease in the central nervous system, the management of older and transplant-ineligible patients, upfront allogeneic transplant and newer agents, and stated that a best practice approach was adopted because the clinical evidence was of lower quality.

Relapsed CCR4+ adult T-cell leukaemia/lymphoma; later PTCL/CTCL

The subclassification proposed by Shimoyama and adopted by the international consensus meetings divides the disease into acute, lymphoma, chronic and smouldering types, using the count of circulating tumour cells, the lactate dehydrogenase, the calcium and the sites involved. It is the first decision and it changes everything: the chronic and smouldering types without unfavourable features are watched, while the acute and lymphoma types are treated at once. The consensus report sets out prognostic factors and a set of response criteria specific to this disease, which is why trials in it are not reported like trials in other lymphomas.

The consensus meeting set out the acute, lymphoma, chronic and smouldering types, the prognostic factors and a set of response criteria specific to the disease, which became the standard reference for trials.

Parathyroid hormone-related protein was found to be abundantly expressed in the blood cells of all 13 patients tested, and in HTLV-1 carriers without symptoms, and the viral Tax protein was shown to switch its gene on.

A cluster of T-cell leukaemias in Kyushu was recognised as a single disease, which led to the search for its cause.